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DSS Case #3. *Grant R. Kolar, M.D., Ph.D., °Terrence Holekamp , MD., Ph.D., *Richard Perrin, M.D., Ph.D. *Division of Neuropathology and °Department of Neurosurgery, Washington University in St. Louis. Disclosure. There are no financial relationships to disclose. Brief History Received.

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dss case 3

DSS Case #3

*Grant R. Kolar, M.D., Ph.D., °Terrence Holekamp, MD., Ph.D., *Richard Perrin, M.D., Ph.D.

*Division of Neuropathology and °Department of Neurosurgery, Washington University in St. Louis

disclosure
Disclosure
  • There are no financial relationships to disclose.
brief history received
Brief History Received
  • 60 year old man transferred after 4 days (presented at 1 day) of worsening confusion and memory deficits
  • PMHX: CAD, MI, HTN
  • Physical Exam: Drowsy and uncooperative, saccadic intrusions of ocular pursuits, right pronator drift, mild bradykinesia
  • Social HX: 8 drinks or more per day
mri flair
MRI (FLAIR)

Compression of 3rd ventricle

Bilateral thalamic T2 hyperintensities with homogeneous enhancement

Scattered nonspecific T2 hyperintensities in cortex

DDX:

Lymphoma

Glioma

Encephalitis

Wernicke’s encephalopathy

CO poisoning

  • PET
    • Mild asymmetric hypermetabolism
    • DDX: lymphoma or high grade glioma
histopathology summary
Histopathology Summary
  • Reactive and mildly proliferative endothelial changes
  • Petechial hemorrhages, minute hemosiderin deposits
  • Mild reactive gliosis and mild edema
  • Microinfarcts w/spheroids, small foamy macrophages, rare red neurons, and sparing of adjacent neurons
slide13
DDx
  • Arterial Infarction (Bithalamic – Artery of Percheron Infarction)
  • Tumor
    • High-grade glioma
    • Lymphoma
  • Toxicity
    • Wernicke’s encephalopathy
    • Carbon monoxide poisoning
    • Hepatic encephalopathy
  • Deep venous system thrombosis
  • Thalamic venous hypertension
followup mra
Followup MRA

Several weeks later …

Showed abnormal venous channel (involving vein of Galen and confluence of sinuses) with arterialized flow

(right common carotid angiogram)

Re-review of MRI noted large vein (originally not reported)

slide15

(Straight Sinus Thrombosed)

Internal Cerebral Veins (paired) and Vein of Galen

Dural Arteriovenous Fistula (dAVF)

Right Occipital Artery

Borden-Shucart Type III tentorialdAVF (Zipfel type 3s)

after embolization and surgical ligation
After Embolization and Surgical Ligation

Red = before treatment; Black = after treatment

answer
Answer
  • Hypertensive Venous Infarction Secondary to a Tentorial Dural Arteriovenous Fistula
dural arteriovenous fistulas
Dural Arteriovenous Fistulas
  • Rare vascular malformation
    • 10-15% of intracranial vascular malformations
  • Abnormal direct connection between dural arteries and cerebral venous system
    • Most appear acquired with:
      • Sinus thrombosis
      • Intracranial venous hypertension
      • Also may result from prior surgery, trauma, or idiopathic causes
    • In contrast arteriovenous malformations (AVMs) are
      • Parenchymal
      • Congenital
experimental etiology of davfs
Experimental Etiology of dAVFs

Other

Venous HTN

Sinus Thrombosis

Retrograde Flow and Enlargement of Veins

Chronic Regional Hypoperfusion

Matrix Metalloproteinase 9

(MMP9)

Induction of VEGF

dAVF Formation

Chen L, Mao Y, et al. Local Chronic Hypoperfusion Secondary to Sinus High Pressure Seems to be Mainly Responsible fo the Formation of Intracranial Dural Arteriorvenous Fistula. 2009. Neurosurgery 64:973

davf clinical presentation
dAVF Clinical Presentation
  • Flow related
    • Pulsatile tinnitis
    • Opthalmological phenomenon
  • Incidental
  • *Intracranial hemorrhage
  • *Non hemorrhagic neurological deficits
    • Often misdiagnosed and given unnecessary procedures
    • Overall 25-55% rate in high grade dAVFs
    • Focal neurological deficits
    • Dementia-like syndrome
      • 20-27% of patients with high grade dAVFs
      • Cortical vs Thalamic patterns
  • *Hemorrhage AND non-hemorrhagic neurological deficits signal high risk dAVFs
    • Carry the same risk of future bleed
imaging of thalamic davfs
Imaging of ThalamicdAVFs
  • Cerebral angiography -- gold standard of diagnosis
    • 100% show multiple supply vessels
    • 90% involve branches of external carotid artery
    • Venous drainage determines level of risk for future hemorrhage
      • Cortical Venous Drainage vs. Direct Sinus Drainage
  • MRI
    • Bithalamic T2 hyperintense signal (100%)
    • Absence of diffusion weighted imaging (DWI) positivity (100%)
    • Peripheral post-contrast enhancement
    • Central hypointensity (hemosiderin deposition)
  • SPECT
    • Abnormal thallium 201 can correspond to venous reflux
    • May have low NAA and elevated lactate
  • PET
    • Reduced cerebral blood flow (venous congestion)
    • Increased oxygen extraction fraction (initially) followed by decrease (cellular death and compensation)
histological features of thalamic davfs
Histological Features of Thalamic dAVFs
  • Acute-subacute anoxic damage
    • Anoxic change in neurons
    • Scattered axonal spheroids
    • Microinfarcts
    • Lipid laden (foamy) macrophages
  • Moderate microvascular response
    • Reactive endothelium
treatment davf
Treatment dAVF
  • Embolization
  • Craniotomy with clip ligation
  • Without treatment, higher grade thalamic dAVFs(reflux into internal cerebral veins) are usually fatal
  • Usually patients demonstrate remarkable progressive cognitive and radiological improvement if treatment is timely.
conclusion
Conclusion
  • Differential diagnosis of bithalamic T2 hyperintensities and lack of DWI signal with cognitive decline must include a thalamic duralarteriovenous fistula
  • Cerebral angiogram is gold standard of diagnosis
  • Histologic features are relatively nonspecific
  • Outcome is correlated with rapid treatment (fatal if untreated)
references
References
  • Chen L, Mao Y, Zhou LF. Local chronic hypoperfusion secondary to sinus high pressure seems to be mainly responsible for the formation of intracranial duralarteriovenous fistula. Neurosurgery64:973-8, 2009
  • Holekamp TF, Murphy RKJ, Kolar GR, Morparia NP, Derdeyn CP, et al. dAVF-Related Thalamic Dementia (DRTD) Syndrome: case series and literature review. Manuscript in progress/submitted.
  • Morparia N, Miller G, Rabinstein A, Lanzino G, and Kumar N. Cognitive decline and hypersomnolence: thalamic manifestations of a tentorialduralarteriorvenous fistula (dAVF). Neurocritical Care 17:429, 2012
  • Rodriguez FJ, Crum BA, Krauss WE, Scheithauer BW, Giannini C. Venous congestive myelopathy: a mimic of neoplasia. Mod Pathol. 18:710-8. 2005
  • Sugrue PA, Hurley MC, Bendok BR, Surdell DL, Gottardi-Littell N, Futterer SF, et al: High-grade dural arteriovenous fistula simulating a bilateral thalamic neoplasm. Clin Neurol Neurosurg 111:629-632, 2009
  • Hurst RW, Bagley LJ, Galetta S, Glosser G, Lieberman AP, TrojanowskiJ, et al: Dementia resulting from duralarteriovenous fistulas: the pathologic findings of venous hypertensive encephalopathy. Am J Neuroradiol 19:1267-1273, 1998